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In page Caroli disease:

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The cause appears to be genetic; the simple form is an autosomal dominant trait, while the complex form is an autosomal recessive trait.[1] Females are more prone to Caroli disease than males.[2] Family history may include kidney and liver disease due to the link between Caroli disease and ARPKD.[3] PKHD1, the gene linked to ARPKD, has been found mutated in patients with Caroli syndrome. PKHD1 is expressed primarily in the kidneys with lower levels in the liver, pancreas, and lungs, a pattern consistent with phenotype of the disease, which primarily affects the liver and kidneys.[1][3] The genetic basis for the difference between Caroli disease and Caroli syndrome has not been defined.[citation needed]